Tone Disorders Research Articles

CAUSES OF PAIN IN THE MUSCLES OF THE LOWER EXTREMITIES IN CHILDREN WITH CEREBRAL PALSY

A high level of muscle spasticity in children with cerebral palsy is one of the causes of degenerative and dystrophic changes in tissues. Against the background of high spasticity, there is a violation of blood flow in the vessels of the lower extremities, which leads to changes in subfascial pressure and the formation of primary pain syndromes. The analysis of the literature shows the need to study the issues related to the mechanism of formation of changes in the muscles and determine the primary causes of pain. Objective. Тo study the mechanism of changes in the muscles of the lower extremities, as well as the primary causes of pain syndrome. Methods. The analysed indicators were obtained during the treatment of 40 patients with cerebral palsy, who were divided into 3 groups depending on the muscle tone disorder. Clinical and instrumental methods were used to examine patients. The data obtained were statistically processed with the determination of M ± m, the coefficient of reliability according to Student’s criteria. Results. According to the results of the study, high spasticity leads to impaired blood flow and increased subfascial pressure in the musculofascial sheaths. There is an inverse correlation between the increase in subfascial pressure, the diameter of the vessel lumen and blood flow velocity. This is one of the causes of degenerative and denervation changes in muscles and fascia. It was proved that the occurrence of primary pain in the lower extremities is caused by various etiological causes: tissue ischemia; increased subfascial pressure; narrowing of pathologically significant anatomical areas in which nerves lie; degenerative and dystrophic changes in the joints. Conclusions. The study found that the cause of changes in the muscles and fascia is a violation of blood flow, a change in subfascial pressure in the musculofascial cases of the lower extremities. The occurrence of pain in the lower extremities in children with cerebral palsy is multifactorial in nature and consists of myofascial, articular, and tunnel syndromes.

Intrathecal baclofen therapy as treatment for spasticity and dystonia: Review of cases in a pediatric palliative care unit

IntroductionPatients managed in the Pediatric Palliative Care Integral Unit (PPCIU) have serious neurological conditions that involve significant damage at central nervous system level. The movement disorder is a very common clinical problem and for the patients where an adequate control of muscle tone is not achieved with usual techniques or drugs, intrathecal baclofen therapy (IBT) should be considered. Materials and methodsDescriptive retrospective study based on the review of clinical records of patients who received IBT being followed by the PPCIU of Madrid Autonomous Region in the timeframe between September 2012 and February 2021. ResultsIBT was implanted in 8 patients affected by infantile cerebral palsy (ICP) with a Gross Motor Function Scale (GMFCS) IV–V, 3 patients was a Pantothenate kinase deficit-associated neurodegeneration (PKAN), 2 had Acquired Brain Damage, and the remaining 3 had, respectively, 2 glutaric aciduria type I (GA-1), and poly-malformative syndrome. In all patients we observed a period of clinical stability after IBT, we call this period “honeymoon”. Two patients died while in the honeymoon period, at 24.9 and 19.6 months from implantation of the pump; the median of duration of the honeymoon period in the remaining 14 was 14.4 months (IQ: 8.3–25.8). ConclusionsIBT was not only used in patients with non-progressive diseases, but also in the group of patients with neurodegenerative or progressive diseases. In all of them, after implantation of the device, we have objectified a period of clinical stability and a better control of muscle tone disorders.

Protocol for combined N-of-1 trials to assess cerebellar neurostimulation for movement disorders in children and young adults with dyskinetic cerebral palsy.

Movement and tone disorders in children and young adults with cerebral palsy are a great source of disability. Deep brain stimulation (DBS) of basal ganglia targets has a major role in the treatment of isolated dystonias, but its efficacy in dyskinetic cerebral palsy (DCP) is lower, due to structural basal ganglia and thalamic damage and lack of improvement of comorbid choreoathetosis and spasticity. The cerebellum is an attractive target for DBS in DCP since it is frequently spared from hypoxic ischemic damage, it has a significant role in dystonia network models, and small studies have shown promise of dentate stimulation in improving CP-related movement and tone disorders. Ten children and young adults with DCP and disabling movement disorders with or without spasticity will undergo bilateral DBS in the dorsal dentate nucleus, with the most distal contact ending in the superior cerebellar peduncle. We will implant Medtronic Percept, a bidirectional neurostimulator that can sense and store brain activity and deliver DBS therapy. The efficacy of cerebellar DBS in improving quality of life and motor outcomes will be tested by a series of N-of-1 clinical trials. Each N-of-1 trial will consist of three blocks, each consisting of one month of effective stimulation and one month of sham stimulation in a random order with weekly motor and quality of life scales as primary and secondary outcomes. In addition, we will characterize abnormal patterns of cerebellar oscillatory activity measured by local field potentials from the intracranial electrodes related to clinical assessments and wearable monitors. Pre- and 12-month postoperative volumetric structural and functional MRI and diffusion tensor imaging will be used to identify candidate imaging markers of baseline disease severity and response to DBS. Our goal is to test a cerebellar neuromodulation therapy that produces meaningful changes in function and well-being for people with CP, obtain a mechanistic understanding of the underlying brain network disorder, and identify physiological and imaging-based predictors of outcomes useful in planning further studies. ClinicalTrials.gov NCT06122675, first registered November 7, 2023.

Advance of research on Hereditary spastic paraplegia type 4

Spastic paraplegia type 4 (SPG4) is the most common type of autosomally inherited spastic paraplegia. Its main clinical features include typical simple hereditary spastic paraplegia, with neurological impairments limited to lower limb spasticity, hypertonic bladder dysfunction, and mild weakening of lower limb vibration sensation, without accompanying features such as nerve atrophy, ataxia, cognitive impairment, seizures, and muscle tone disorders. SPAST is the main pathogenic gene underlying SPG4, and various pathogenic SPAST variants have been discovered. This disease has featured a high degree of clinical heterogeneity, and the same pathogenic variant can have different age of onset and severity among different patients and even within the same family. There is a lack of systematic research on the correlation between the genotype and phenotype of SPG4, and the pathogenic mechanism has remained controversial. This article has provided a review for the clinical characteristics, pathogenic gene characteristics, correlation between the genotype and phenotype, and pathogenic mechanism of this disease, with an aim to provide reference for its clinical diagnosis and treatment.

Management of Cerebral Palsy through Siddha System of Medicine - A Review

Cerebral palsy is a disorder of movement, muscle tone and posture. Cerebral palsy is due to abnormal brain development often before birth. Cerebral palsy is the most common physical disability in children; about 2 per 1000 live-born are later diagnosed with CP. Social inequality in risk has been reported for outcomes strongly associated with CP, including preterm birth, low birth weight and intrauterine growth restriction. In medical world, there are a lot of newly developed technologies are present in children health for pediatric care. Therefore several generations before in Siddha literatures are described the child growth, child growing period and the specific group of medicinal plants suitable for pediatric population and the diseases that occur in various stages of childhood and their treatment. In Siddha literature, most of the symptoms of Cerebral Palsy are correlated to Siravatham, Sirasthamba Vatham. I reviewed a complete manual on siddha treatment for cerebral palsy. The external therapies are like Varmam, Podithimirthal, Ennaiooral (Sira Pootchu), Ennaiozhukku (Tharai), Thokanam, Nasiyam. One of the concepts of Siddha system is diseases are raised from impairment of Mukkutram (Vatham, Pitham and Kabam).These external therapies were found to reduce Vadha and Kabha and induce Pitha. So that the above therapies are used to reduce the symptoms and manage the condition.

Individual perception of environmental factors that influence lower limbs spasticity in inherited spastic paraparesis

Phenotypic variability is a consistent finding in neurogenetics and therefore applicable to hereditary spastic paraparesis. Identifying reasons for this variability is a challenge. We hypothesized that, in addition to genetic modifiers, extrinsic factors influence variability. Our aim was to describe the clinical variability in hereditary spastic paraparesis from the person's perspective. Our goals were to identify individual and environmental factors that influence muscle tone disorders and derive interventions which could improve spasticity. This study was based on self-assessments with questions on nominal and ordinal scales completed by participants with hereditary spastic paraparesis. A questionnaire was completed either in-person in the clinic or electronically via lay organization websites. Among the 325 responders, most had SPG4/SPAST (n=182, 56%) with a mean age at onset of 31.7 (SD 16.7) years and a mean disease duration of 23 (SD 13.6) years at the time of participation. The 2 factors identified as improving spasticity for > 50% of the responders were physiotherapy (193/325, 59%), and superficial warming (172/308, 55%). Half of the responders (n=164, 50%) performed physical activity at least once a month and up to once a week. Participants who reported physiotherapy as effective were significantly more satisfied with ≥ 3 sessions per week. Psychologically stressful situations (246/319, 77%) and cold temperatures (202/319, 63%) exacerbated spasticity for most participants. Participants perceived that physiotherapy reduced spasticity and that the impact of physiotherapy on spasticity was much greater than other medical interventions. Therefore, people should be encouraged to practice physical activity at least 3 times per week. This study reported participants' opinions: in hereditary spastic paraparesis only functional treatments exist, therefore the participant's expertise is of particular importance.

Assessment of postural balance in patients with stroke

The Cerebral Vascular Accident (CVA) results in disabilities of paretic limbs, but few studies have investigated the impacts of CVA on perception deficits as well as related functional changes. Motor function alterations caused by CVA, such as the presence of associated reactions, loss of postural control mechanisms, and tone disorders, shift the body's center of gravity, generating a risk of falls. The aim of the present study was to evaluate postural balance changes in patients affected by CVA using the POMA-Brazil Scale (Performance Oriented Mobility Assessment). A cross-sectional study was conducted to assess postural balance changes in patients diagnosed with CVA undergoing rehabilitation at the UNIT Health Center, CEMISE Physiotherapy Service, and Maria Virgínia Leite Franco Rehabilitation Center in Aracaju, Sergipe. The B-POMA subscale consists of 13 tasks (sitting balance, rising from a chair, immediate standing balance, standing balance, eyes closed balance, 360-degree turning balance, resistance to displacement (Nudge Test), neck turning, unipedal stance balance, spinal extension, reaching upward, leaning forward, and sitting), aimed at detecting fall risk factors in elderly individuals with disabilities or chronic illnesses. Descriptive statistics were used for numerical variables, frequency distribution for demographic and clinical variables, and individuals per task, with the application of the chi-square test and the Student's t-test. Among the tasks measured according to the B-POMA subscale, sitting balance was the most frequent qualitative response (97.5%), followed by immediate standing, eyes closed, and leaning forward. Unipedal stance balance was the least common normal task (25%), and 360-degree turning was the most adaptive (62.5%). Balance changes are associated with cerebral vascular accidents in terms of dynamic equilibrium. This study demonstrated that unipedal stance balance was the most affected task, 360-degree turning balance was the most adaptive, and sitting balance was the least affected. There were no significant differences in balance scores based on sex, age, and time of injury.

Problems of physical rehabilitation of movement disorders in the pathology of the hip joint in patients with the consequences of a cerebral stroke

The purpose of the work was to analyze the experience of foreign specialists regarding movement and structural disorders of the proximal part of the femur in patients with the consequences of stroke and their impact on the effectiveness of rehabilitation programs for hip joint pathology. To fulfill the goal, an analysis of foreign literary sources was carried out in modern databases: Google Scholar, Web of Science, PubMed, Medline, Cochrane, Pedro. The search depth is 22 years (from January 2000 to October 2021). The analysis of literature sources indicated that limitation of motor activity and axial loads in patients after acute cerebrovascular accident, in particular limitation of mobility in the hip joint, impairment of muscle strength and tone due to hemiparesis can lead to the development of osteoporosis. Particularly important from the point of view of adverse consequences is a decrease in mineral density and fractures of the proximal part of the femur. On the other hand, disturbed biomechanics of movements in the hip joint in persons with the consequences of a cerebral stroke, under the condition of axial load on the limb, can lead to the development of osteoarthritis of the hip joint. Osteoarthritis results in limited mobility of the hip joint, which leads to secondary immobilization of the hip joint and, as a result, can also lead to further osteoporosis. Existing disorders of neuromuscular control, muscle tone and strength, limitation of mobility of the hip joint, including due to spasticity, problems with static and dynamic balance, and, as a result, an increased risk of falling in patients with the consequences of a stroke, can negatively affect the recovery of motor functions, activity limitation, and, as a result, reduce the effectiveness of rehabilitation of patients after hip joint replacement. Existing movement disorders, as a result of a stroke, require the development and justification of new rehabilitation programs, including the use of modern technical means and virtual reality.

ВЫРАЖЕННОСТЬ ЭНДОТЕЛИАЛЬНОЙ ДИСФУНКЦИИ КАК ОБЪЕКТИВНЫЙ КРИТЕРИЙ СТЕПЕНИ ТЯЖЕСТИ ПРЕЭКЛАМПСИИ

Preeclampsia remains one of the most significant obstetric problems due to its high prevalence, high incidence of maternal and perinatal morbidity and mortality, as well as the need for significant economic costs for the management and delivery of pregnant women with this pathology. In recent years, a number of authors have proven the most important role of vascular endothelial dysfunction in initiating disorders of vascular tone and blood coagulation potential, which underlie the formation of multiple organ failure in pregnant women with preeclampsia. The aim of the study was to establish new objective criteria for assessing the severity of preeclampsia based on the study of the functional state of the vascular wall by various methods, both invasive and non-invasive. 118 women with 28-37 weeks of pregnancy were examined. The 1st observation group consisted of 58 pregnant women with moderate preeclampsia, the 2nd observation group included 20 pregnant women with severe preeclampsia. The control group consisted of 40 women with a physiological course of pregnancy. The functional activity of the vascular wall was assessed by the level of endothelin-1, thrombospondin, intercellular adhesion molecules and nitric oxide metabolites in the blood by enzyme immunoassay. In patients with moderate preeclampsia, the content of nitric oxide metabolites significantly decreased, and the content of endothelin-1, thrombospondin, and intercellular adhesion molecules increased relative to patients in the control group. These changes were significantly more pronounced in patients with severe preeclampsia. At the same time, to study the function of the endothelium in pregnant women with preeclampsia, we used a non-invasive method - an ultrasonic cuff test according to the Celermajer D.S. In patients with moderate preeclampsia, the degree of endothelium-dependent arterial expansion during the cuff test decreased by more than 2 times compared with the parameters of pregnant women in the control group. In pregnant women with severe preeclampsia of the group, an even more pronounced decrease in the degree of dilatation of the brachial artery was noted. Thus, the determination of markers of endothelial dysfunction in the blood, as well as the use of an ultrasonic cuff test, made it possible to identify violations of the vasomotor function in pregnant women with preeclampsia, which correlate with the severity of preeclampsia. The use of an ultrasonic cuff test and the determination of endothelial dysfunction markers can be recommended as additional objective diagnostic criteria for the severity of preeclampsia, the transition from moderate preeclampsia to severe preeclampsia.

Acute Bilirubin Encephalopathy: A Propos of 151 Cases Collected during a Multicentric Study in Senegal

Introduction: Bilirubin encephalopathy is a debilitating complication of severe neonatal unconjugated bilirubin jaundice. The aim of this study was to determine the frequency of occurrence of this complication and to describe the diagnostic and prognostic aspects in Senegal. Materials and Methods: This was a multicenter, descriptive and analytical study conducted in 32 neonatal units of various levels, with retrospective (January to December 2020) and prospective (January to August 2021) collection. All neonates hospitalized for acute bilirubin encephalopathy were included. The diagnostic criteria were the presence of jaundice associated with neurological signs (muscle tone disorders, abnormal movements, convulsions, etc.), with no other obvious etiology found. The data were entered and analyzed using SPSS version 23 software. The significance threshold was retained for a p-value Results: We registered 151 patients. The mean age was 6 days and the sex ratio was 2.5 (clear male predominance). The majority of newborns were from Dakar (51%). Twenty-two (22%) were premature and 32% had low birth weight. Half of the newborns (50%) came from home and 87% were exclusively breastfed. The average time to consult was 3 days. Neurological signs were dominated by decreased primitive reflexes (74%), abnormal movements (59%) and lethargy (50%). Dehydration (30%) and anemia (26%) were often associated. The mean bilirubin level was 416 umol/l. Neonatal infections (19%) and Rhesus (16.7%) and ABO (8.7) incompatibility were the main causes. One third (33%) of patients had received intensive phototherapy and only 2% had received exchange blood transfusion. The case fatality was 48.9%. Conclusion: Bilirubin encephalopathy remains a major concern in Senegal. It is associated with high mortality and numerous neurological sequelae. Progress in terms of early detection and appropriate management is urgently needed on a national scale.

Level of Awareness and Attitude Toward Cerebral Palsy Among Parents in Al-Baha City, Saudi Arabia.

Background Cerebral palsy (CP) is a chronic disorder of motion, posture, and tone which occurs due to brain insult during the period of brain growth. It is a disabling disorder in both motor and intellectual aspects.Fortunately, CP is a manageable disease that can be managed in part by increasing the knowledge and understandingof the parents. Methodology This cross-sectional, prospective, community-based study aimed to assess the level of parents' knowledge and their attitude toward CP using an electronic questionnaire. The parents' knowledge was classified as good or poor based on an adopted scoring system. The parents' attitude was categorized as positive or negative. Results Our study results showed that good knowledge (those with a score more than 60% of the total score) was noted in 275 (61.1%) participants, whereas 175 of the participants had poor knowledge (38.9%), especially regarding awareness of the diagnosis of CP. Conclusions Most participants had an overall good knowledge of CP although they had insufficient knowledge of some aspects of the disease such as causes, disease course, clinical presentations, diagnosis, and prognosis. Although the results showed a positive attitude concerning playing with a child with CP, unfortunately, there was a negative attitude toward hiring a CP patient and a strongly negative attitude toward marrying a patient with CP.

Analysis of the prevalence of asymmetry and muscle tone disorders in the first year of life among youth with idiopathic scoliosis: A retrospective case-control study.

Not much is known about developmental motor disorders in the first year of life of children diagnosed with idiopathic scoliosis (IS). This study aimed to compare the occurrence of asymmetry or muscle tone disorders in the first year of life in adolescents with IS and their healthy counterparts. The parents of adolescents with IS and without scoliosis completed a survey on the occurrence of asymmetry, abnormal muscle tone, and physiotherapy in their children in the first year of life. Pearson's chi square test and Cramer's coefficient were used. The final analysis included 527 surveys completed by parents of adolescents with idiopathic scoliosis (150) and without scoliosis (377). A significantly higher frequency of asymmetry (p= 0.001) and muscle tone disorders (p= 0.001) was noted in adolescents with idiopathic scoliosis. The results also revealed a significant association between scoliosis and asymmetry (p= 0.001), as well as muscle tone (p= 0.001). Developmental asymmetry or improper muscle tone in the first year of life could be considered a potential factor in the development of scoliosis; however, this hypothesis should be confirmed in future studies. Infants diagnosed with developmental disorders require systematic observation.

Mobile application for monitoring and normalizing the mastication muscle functional condition

Relevance. We have developed and patented a mobile application for monitoring and normalizing the mastication muscle functional state to solve the problems of detecting people with suspected dysfunction of the jaw muscles and increase the efficiency of muscle exercises.Purpose. To evaluate the positive effect of the mobile application to control and normalize the mastication muscle functional state in the clinical orthodontic practice.Material and methods. The study included 74 patients of Children Clinical Dental Clinic No. 2 of Voronezh, aged 6 to 12 years, who presented for a dental check-up. The masseter muscle tone was determined using the Mioton-3S device.Results. The authors’ method of self-examination does not give a systematic overestimation or underestimation of parameters (p = 0.083) compared to the standard myotonometry, which evidences its reliability and the possibility to use for preliminary examination and detection of people with suspected abnormal muscle tone. The analysis of the masseter muscles’ tone one month later revealed a statistically signifcant (p = 0.034) decrease in the number of patients with tone disorder among the mobile application users compared to the patients who did not use the application.Conclusion. The developed mobile application is efficient for preliminary assessment and detection of people with suspected abnormal muscle tone. Mobile application helps increase efficiency and awareness of muscle exercises.

Intrathecal baclofen therapy as treatment for spasticity and dystonia: Review of cases in a pediatric palliative care unit

IntroductionPatients managed in the Pediatric Palliative Care Integral Unit (PPCIU) have serious neurological conditions that involve significant damage at central nervous system level. The movement disorder is a very common clinical problem and for the patients where an adequate control of muscle tone is not achieved with usual techniques or drugs, intrathecal baclofen therapy (IBT) should be considered. Materials and methodsDescriptive retrospective study based on the review of clinical records of patients who received IBT being followed by the PPCIU of Madrid Autonomous Region in the timeframe between September 2012 and February 2021. ResultsIBT was implanted in 8 patients affected by infantile cerebral palsy (ICP) with a Gross Motor Function Scale (GMFCS) IV–V, 3 patients was a Pantothenate kinase deficit-associated neurodegeneration (PKAN), 2 had Acquired Brain Damage, and the remaining 3 had, respectively, 2 glutaric aciduria type I (GA-1), and poly-malformative syndrome. In all patients we observed a period of clinical stability after IBT, we call this period “honeymoon”. Two patients died while in the honeymoon period, at 24.9 and 19.6 months from implantation of the pump; the median of duration of the honeymoon period in the remaining 14 was 14.4 months (IQ: 8.3–25.8). ConclusionsIBT was not only used in patients with non-progressive diseases, but also in the group of patients with neurodegenerative or progressive diseases. In all of them, after implantation of the device, we have objectified a period of clinical stability and a better control of muscle tone disorders.

Correction of myofunctional disorders in children with special needs

The article presents myofunctional therapy in young children with special needs. The literature is analyzed and it is shown that this problem is quite rare. It is shown that the influence of anomalies in the structure and functions of the dental system on the formation and development of the musculoskeletal system of the maxillofacial region and the speech-communication mechanism in children). The reasons for the appearance of myofunctional disorders in children are presented. The causes of myofunctional disorders are: dysfunction; bad habits; genetic predisposition.
 Indications for the correction of disorders of muscle tone of masticatory and facial muscles have been established. Groups of manifestations of myofunctional disorders in children of primary school age are characterized. The following groups of manifestations of myofunctional disorders have been identified: group I – myofunctional disorders caused by biological determinants; group II – myofunctional disorders caused by social determinants; group III – myofunctional disorders caused by biosocial determinants.
 Systems of work on correction of disorders in children are presented. A comparative quantitative and qualitative analysis of the positive dynamics in overcoming the manifestations of disorders of facial expressions, oral, articulatory praxis and physiological speech breathing is given. The system of work on the correction of myofunctional disorders in children of primary school age with special educational needs. It is proved that the complex application of kinesiotaping, orofacial and articulation massage, myogymnastics and long-term wearing of special trainers in the complex rehabilitation of myofunctional disorders in primary school children with special educational needs helped regulate the tone of facial expressions, chewing gum, and strength; reduction of hypersalivation; normalization of mobility of jaw joints, lower jaw, larynx; partial qualitative improvement of chewing and swallowing processes; reduce swelling and congestion in the muscles.

Efektifitas Massage Deep Friction dan Latihan Stabilisasi Trunk Pada Cerebral Palsy

Cerebal Palsy is a permanent disorder in the development of movement and non-progressive body posture in the form of muscle tone abnormalities that cause postural tone disorders in the form of spasticity, especially in the legs, sitting balance so that the patient's daily functional activities are disrupted. This study aims to determine the effect of deep friction massage and trunk stabilization exercises on sitting balance and spasticity in cerebral palsy patients. This type of research is a pre-experiment with a one-group pre-test-post-test design. It was held at the Makassar special school from August to October 2018, with a research sample of 17 cerebral palsy patients. Sitting balance data was obtained using a pediatric balance scale and leg spasticity data was obtained using the Asworth scale. The results of the Paired sample T Test obtained p = 0.000 for spasticity and sitting balance with p = 0.000 after being given deep friction massage and trunk stabilization exercises. Thus deep friction massage and trunk stabilization exercises are effective in improving sitting balance and spasticity in cerebral palsy patients.

Effects of Kinesio taping on forearm supination/pronation performance fatigability

BackgroundRepetitive exertion in supination/pronation could increase the risk of forearm diseases due to fatigue. Kinesio taping (KT) is a physical therapy technique that decreases muscle tone and musculoskeletal disorders (MSDs) risk. Many assumptions about taping have been made and several studies have considered the taping applications; however, the effect of KT on strength and fatigue of the forearm supination/pronation remains unclear. The purpose of this study was to evaluate the effect of KT on forearm performance fatigability.MethodsA screwing test was constructed to measure the forearm force loss and screwing efficiency during repetitive supination/pronation. Data from 18 healthy adults who underwent both KT and no taping (NT) sessions were used to investigate the forearm strength change in terms of grip force (GF), driving torque (DT), and push force (PF). The maximal isometric forces before and after the screwing test and force decreasing rate (efficiency) during screwing were evaluated to assess the performance fatigability in KT and NT conditions.ResultsA statistically significant force loss (FL) in maximal isometric GF (p = 0.039) and maximal isometric DT (p = 0.044); however, no significant difference was observed in maximal isometric PF (p = 0.426) between NT and KT. KT provides greater screwing efficiency than NT.ConclusionsKT could not improve FL in the maximal muscle strength of the forearm in healthy subjects. KT on the forearm was associated with a lesser decline in DT efficiency than NT, implying that KT could decrease the loss rate of muscle strength and delay the development of fatigue; however, the KT did not yield improvements in PF while performing screwing tasks.

Dose selection of Incobotulinumtoxin A for the treatment of spasticity and sialorrhea in cerebral palsy: results of a retrospective multicenter study

Introduction. In patients with infantile cerebral palsy (CP), botulinum therapy is used to treat both muscle tone disorders and sialorrhea. Therefore, it is logical to use one preparation of botulinum toxin type A to treat spasticity and sialorrhea in one injection procedure. The aim of the work is to conduct a retrospective analysis of data from 15 centres that treat patients with cerebral palsy and use the botulinum therapy method to determine the optimal doses of IncobotulinumtoxinA (IBTA) for the treatment of spasticity and chronic sialorrhea in real clinical practice. Materials and methods. The treatment results of 389 children with cerebral palsy (including 211 (54.2%) boys) with IBTA were analyzed. The majority were children with bilateral forms of cerebral palsy - 312 (80.2%). The average age of the patients was 5.27 ± 3.71 years, the average weight of the patients was 18.8 ± 10.9 kg. Results. The total dose of IBTA in the group of 389 patients with cerebral palsy for the treatment of spasticity was 163.74 ± 80.65 U (25-550; 95% CI 155.7-171.7) and 10.4 ± 5.4 U/kg body weight (1,25-29.7; 95% CI 9.8-10.9). The total dose of IBTA in the group of patients with cerebral palsy with simultaneous treatment of spasticity and chronic sialorrhea (n = 16) was significantly higher: 267.18 ± 124.57 U (115-570; 95% CI 200.8-333.6) and 13, 0 ± 7.1 U/kg (5.8-24.6; 95% CI 9.2-16.8). In the lower extremities, the most frequent target muscles were the gastrocnemius (55.0% of cases; 95% CI 49.9-60.0) and semitendinosus / semimembranous muscle (46.3% of cases; 95% CI 41.2-51.4 ), and in the upper limbs - pronator teres (48.6% of cases; 95% CI 43.5-53.7) and biceps brachii (28.8% of cases; 95% CI 24.3-33.6). Limitations of the study. The limitations of our work are the use of an open retrospective study format, a relatively small sample of patients with chronic sialorrhea, the absence of long-term follow-up of patients and the results of repeated IBTA injections. Conclusion. If it is necessary to use botulinum therapy for the treatment of spasticity and sialorrhea in a child with CP, it is optimal to use the product IncobotulinumtoxinA, which will allow correction of two pathological manifestations in one procedure and can shorten the intervals between repeated injection cycles.

Hyperekplexiа. Clinical observation

The differential diagnosis of paroxysmal conditions, as well as disorders of muscle tone (hypertension) in the neonatal period and in young children is quite complicated. Various states of the nervous system in newborns are transient and permanent, optimal and suboptimal, normal and pathological. Among them, we can mention non-epileptic paroxysmal states of early childhood. In some cases, non-epileptic paroxysmal states of early childhood is accompanied by motor disorders, manifested by an excessive increase in limb tone in newborns. This pathological condition of muscle tone in the English-language literature is referred to by the term stiffness baby (the syndrome of a rigid or fettered baby). Neonatal pathological muscle hypertonicity, unlike physiological hypertonicity of muscles of a newborn, is a rather rare condition. The article presents literature data and a description of the clinical observation of a patient with hyperekplexia. Hyperekplexia is a rare paroxysmal movement disorder in young children. The main clinical variants of the disease, methods of diagnosis and correction, the main mutations associated with this condition are considered. The article describes the own clinical observation of an early-age patient with hyperekplexia, its clinical picture, features of paroxysmal states and therapy, neuroimaging data, electroencephalographic phenomena recorded in the patient and genetic testing that confirmed the diagnosis of non-epileptic paroxysmal disorders. The child has a mutation in the ATAD1 gene associated with type 4 Hyperekplexia (618011).

Les séquelles motrices et sensorielles du neuropaludisme chez l’enfant à Kinshasa, bilans et perspectives de prise en charge

ObjectiveIdentify and evaluate the severity of post-neuropaludism motor and sensory sequelae in children in Kinshasa, the scales used to quantify them, and the effective rehabilitation techniques used to manage them. MethodologyThis is a multicenter, transversal and descriptive study, carried out in Kinshasa, during the period from July to September 2019. It consisted in evaluating the frequency and severity of motor and sensory sequelae in children aged 0 to 15 years, within three weeks of an episode of neuropaludism with sequelae. Prior informed consent had been obtained from their parents. ResultsThe frequency of post-neuropaludism motor and sensory sequelae in Kinshasa was 33%. Spastic hypertonia was the most objective tone disorder in 78.8% of patients. Dysarthria and aphasia were the most objective language disorders in 42.4 and 24.2% of cases. Late consultation (87.0%) and convulsions (78.3%) significantly influenced the severity of hypertonia and severity of sequelae. In 93.9% of the cases, no evaluation scale was applied in patients undergoing rehabilitation. Only in patients of the university hospital of Kinshasa (6.1%), the scales were realized and benefited from the protocol including all other rehabilitation techniques according to our perspectives, with a satisfactory evolution. ConclusionPost-neuropaludism motor and sensory sequelae are present in Kinshasa but are very insufficiently evaluated, with inadequate management increasing their severity. So, the need for a standard protocol, advocating prior assessments through specific measurement scales as well as early and multidisciplinary management.